open access

Vol 47, No 6 (2013)
ARTYKUŁ ORYGINALNY
Submitted: 2012-09-19
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Does brain degeneration in Wilson disease involve not only copper but also iron accumulation?

Marta Skowrońska, Tomasz Litwin, Karolina Dzieżyc, Agata Wierzchowska, Anna Członkowska
DOI: 10.5114/ninp.2013.39071
·
Neurol Neurochir Pol 2013;47(6):542-546.

open access

Vol 47, No 6 (2013)
ARTYKUŁ ORYGINALNY
Submitted: 2012-09-19

Abstract

Background and purpose

Wilson disease (WD) is an autosomal recessive inherited disorder of hepatic copper metabolism. Clinical manifestations of WD include neurologic, hepatic and psychiatric symptoms. Most WD patients with the neuropsychiatric form, and some with the hepatic and presymptomatic forms have both hypointense and hyperintense lesions in basal ganglia on T2-weighted magnetic resonance imaging (MRI), which can be iron and copper accumulation. It has been established that T2* and susceptibility-weighted imaging (SWI) are highly sensitive in demonstrating brain iron accumulation, showing decreased signal intensity. Hypointense globus pallidus (GP) signal has been described on T2-, T2*-weighted images and on SWI as typical MRI lesion for patients with neurodegeneration with brain iron accumulation (NBIA). We investigated whether WD patients have MRI changes suggesting iron accumulation using T2*-weighted and VEN_BOLD SWI imaging protocols.

Material and methods

Standard MRI with additional sequences (T2*-weighted and VEN_BOLD SWI) was performed in consecutively admitted, clinically stable, and treated patients.

Results

Twenty-eight patients entered the study. Hypointensity in the GP was observed on T2*-weighted images in 10 patients. Using the VEN_BOLD SWI technique, we found hypointense signal in GP in 20 patients.

Conclusions

MRI data suggest not only copper but also iron accumulation in GP in WD patients.

Abstract

Background and purpose

Wilson disease (WD) is an autosomal recessive inherited disorder of hepatic copper metabolism. Clinical manifestations of WD include neurologic, hepatic and psychiatric symptoms. Most WD patients with the neuropsychiatric form, and some with the hepatic and presymptomatic forms have both hypointense and hyperintense lesions in basal ganglia on T2-weighted magnetic resonance imaging (MRI), which can be iron and copper accumulation. It has been established that T2* and susceptibility-weighted imaging (SWI) are highly sensitive in demonstrating brain iron accumulation, showing decreased signal intensity. Hypointense globus pallidus (GP) signal has been described on T2-, T2*-weighted images and on SWI as typical MRI lesion for patients with neurodegeneration with brain iron accumulation (NBIA). We investigated whether WD patients have MRI changes suggesting iron accumulation using T2*-weighted and VEN_BOLD SWI imaging protocols.

Material and methods

Standard MRI with additional sequences (T2*-weighted and VEN_BOLD SWI) was performed in consecutively admitted, clinically stable, and treated patients.

Results

Twenty-eight patients entered the study. Hypointensity in the GP was observed on T2*-weighted images in 10 patients. Using the VEN_BOLD SWI technique, we found hypointense signal in GP in 20 patients.

Conclusions

MRI data suggest not only copper but also iron accumulation in GP in WD patients.

Get Citation

Keywords

Wilson disease, magnetic resonance imaging, copper, iron

About this article
Title

Does brain degeneration in Wilson disease involve not only copper but also iron accumulation?

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 47, No 6 (2013)

Pages

542-546

DOI

10.5114/ninp.2013.39071

Bibliographic record

Neurol Neurochir Pol 2013;47(6):542-546.

Keywords

Wilson disease
magnetic resonance imaging
copper
iron

Authors

Marta Skowrońska
Tomasz Litwin
Karolina Dzieżyc
Agata Wierzchowska
Anna Członkowska

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